Observational studyOrphanet journal of rare diseases2014
The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome.
Observational study in Orphanet journal of rare diseases, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 39 citations in OpenAlex.
- Latent Vasculopathy of a Peripheral Pulmonary Artery in FBN1-Related Marfan Syndrome.Pathology international · 2026Article
- Autograft Enlargement After the Ross Procedure in Pediatric Patients: Somatic Growth or Pathologic Dilatation?World journal for pediatric & congenital heart surgery · 2026Review
- Ross Procedure After Valve-Sparing Root Replacement With Marfan Syndrome.JACC. Case reports · 2026Article
- Magnetic Resonance Imaging of Temporomandibular Joint and Aortic Root Score in Fibrillinopathies.Medicina (Kaunas, Lithuania) · 2024Article
- Pulmonary Artery Dilatation Due to Pressure or Volume Overload in Congenital Heart Disease.Journal of clinical medicine · 2024Article
- Cardiac dimensions and hemodynamics in healthy juvenile Landrace swine.Cardiovascular ultrasound · 2024Article
- Longitudinal follow-up by MR angiography reveals progressive dilatation of the distal aorta after aortic root replacement in Marfan syndrome.European radiology · 2023Article
- Assessment of aortic diameter in Marfan patients: intraindividual comparison of 3D-Dixon and 2D-SSFP magnetic resonance imaging.European radiology · 2023Article
- Understanding Pulmonary Autograft Remodeling After the Ross Procedure: Stick to the Facts.Frontiers in cardiovascular medicine · 2022Review
- Magnetic resonance angiography derived predictors of progressive dilatation and surgery of the aortic root in Marfan syndrome.PloS one · 2022Article
- Cardiovascular manifestations in Marfan syndrome.Medicine and pharmacy reports · 2021Article
- Pulmonary artery aneurysm as a result of group 2 pulmonary artery hypertension in a patient with significant mitral and aortic valve disease - review and case report.Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgery · 2020Review
- Reliability of non-contrast magnetic resonance angiography-derived aortic diameters in Marfan patients: comparison of inner vs. outer vessel wall measurements.The international journal of cardiovascular imaging · 2020Article
- Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta.International journal of molecular sciences · 2020Review
- Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome.Journal of clinical medicine · 2019Article
- Differences in Cardiovascular Manifestation of Marfan Syndrome Between Children and Adults.Pediatric cardiology · 2019Article
- The Pulmonary Artery in Pediatric Patients with Marfan Syndrome: An Underestimated Aspect of the Disease.Pediatric cardiology · 2018Article
- Cardiovascular Management of Adults with Marfan Syndrome.European cardiology · 2016Review
- Pulmonary artery diameters, cross sectional areas and area changes measured by cine cardiovascular magnetic resonance in healthy volunteers.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2016Article
- Automatic segmentation and analysis of the main pulmonary artery on standard post-contrast CT studies using iterative erosion and dilation.International journal of computer assisted radiology and surgery · 2016Article
Corrections and comments
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Authors and funding
19 authors at 7 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEchocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS).
methodsWe enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm.
resultsMPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86-1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P < .001), but ratios >1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters.
conclusionsWe established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP.
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